Less HLH; More Hope

The good news is this: it looks like Scooby is on target for a bone marrow transplant in the next few weeks. His blood levels are doing what they are supposed to be doing; the chemo seems to have destroyed any evidence of HLH in his system; his temperature has been normal and any infections are being quickly recognised and dealt with; he is less sleepy and a little more ‘with it’ than he has been for the last few weeks; and so far the tests for Rocky have been clear to show that he qualifies as a suitable donor. The doctors had to keep the steroids raised higher than they wanted to in order to get the levels right, and his various drips and infusions are being constantly tweaked to make sure he’s getting what he needs. His appetite has been almost normal this week and his weight is now not much under what it was when he first went in, which is pretty surprising.

And one really REALLY weird thing has happened – his double vision has gone. Since mid-January 2010 he’s seen two of everything, and the angle has shifted when the disease in his brain has got worse, but he’s never had a day without it, until last week. It’s actually quite freaky to see him looking at us face on because we’d grown so used to one eye pointing off in another direction. We have no idea why this has happened – it’s possible that the steroids have caused some inflammation to go down, or caused something else to move over (his face on the outside seems to be getting bigger every day and around his eyes it's very puffy, so there could be something similar going on in the inside) so it could just be a temporary thing, but as Richard said, in the beginning it was the only thing that seemed to be wrong (and we wouldn’t have taken him to hospital if it wasn’t for that), and now it’s the one thing that seems to have gone right! 

So, while we are pleased that the doctors are happy that his body is finally following The Plan, we are also slightly dazed by realising all that a bone marrow transplant entails. The transplant itself is a ridiculously easy procedure (once you have a donor, which has been a breeze for us thanks to our over-zealous baby making, but often involves a worldwide quest for other recipients), because it is simply sucking some cells from the donor, putting them into a bag, and then dripping them through his central line for a couple of hours, like the half dozen blood transfusions he’s had already. The complexity of what his body has to go through, however is pretty mind-blowing, life-changing and scary stuff. Before the transplant, he will have chemo that destroys all his own bone marrow within a few days. So there will be no immune system and no ability to create any kind of blood cells at all. When Rocky’s stem cells are put into his body, they have to find the right places to go, settle into those places, and then start to do the jobs they are supposed to do, while multiplying themselves enough times until his body has enough bone marrow to be able to reproduce blood cells and immunity for his whole system. This will take about six months in all, and so in that time he will be restricted in what he can eat and where he can go, and will still need pretty heavy medications, including more blood transfusions and anti-rejection drugs. His central line will stay in for a long time so they can give him those, and coming home depends on a massive range of factors. In fact, when patients come home, the hospital keeps their bed assigned to them for a while afterwards, because chances of complications are high and an infection or an alteration in blood levels can mean they are back in for days or weeks until it is sorted.

The minimum stay after a BMT is six weeks, and we’re pretty sure that as Scooby has NEVER been a straightforward case and has needed so many factors to keep him stable this year (even on this ward, the nurses are surprised at how many medications he is on!) that he will be in for longer than that. So as well as the implications of how tricky it’s going to be to get his new immunity and blood-making systems to work effectively without succumbing to any dangers on the way, we’re also dealing with the logistics of knowing that we will be physically divided as a family for a long time still yet. When a transplant was a hazy mirage in the distance I was girding myself to be ready until perhaps the end of March (I don’t know why – that’s about as far into the future as I could think at that point). Now that we can see what it looks like, we are peering into April and May, which includes my cousin’s wedding, the birth of our new nephew, and my sister and brother-in-law leaving to travel and do missions for a year, and knowing that even if he’s out before the summer, we will still be under the no-germs, restricted food, restricted visitors, etc, etc, regime until the autumn.

So we have been slowly processing this over the last week and coming to terms with the idea. The idea of most of this year being absorbed with Scooby’s treatment. And of course, the idea that by the end of the year, we may possibly have said goodbye to this horrible disease that has been doing its best to slowly steal him. I can hardly dare to imagine it, but IF the treatment has got rid of his mystery two-year illness as well as the HLH and IF the post-transplant obstacles are overcome, then we get to keep him. What permanent damage has already been caused by the disease and the intensive medication, we don’t yet know, but four weeks ago I didn’t think he was ever going to come home again, so at this stage I don’t even care about that. We have hope – not just a tiny glimpse of it, but a big dollop of it that comes with a name and a shiny booklet and a six month plan. Hooray for hope.


  1. My friends little boy had Fanconi Anemia. Reading through your words this morning reminded me of my many conversations with her. Her son was so very poorly. They had another baby, she was totally clear of Fanconi. They used her cord blood to treat Nathan. That was about 5 years ago. They too followed 'your plan' - he is at High school now and doing well. I know that your precious little man is going to be well again. As I have said before 'once you have a plan you can take action!!' I am overjoyed for you - sat here with a massive daft grin on my face. xxxxx love, as always, from Shabbs xxx

  2. Goodmorning........oh this is indeed very very wonderful news to wake up to. My heart aches for you all, but now you have The Plan you do indeed have something very uplifting and positive. There is now a very bright light at the end of your tunnel. Bless you all. xx
    Triplets .

  3. can't stop crying.
    can't stop smiling.
    can't stop praising.
    can't stop thanking ..... from the deepest place in the bottom of my heart.
    SO want to hug you Esther - a great big rejoicing hug.

    now I have to go and jump up and down a little bit

  4. Proverbs 29:18 Where there is no revelation/plan, people cast off restraint; but blessed is the one who heeds wisdom’s instruction/little booklet.

    Dead excited can't wait to tell our church.

    xx Doreen xx

  5. I heard a Pastor teach yesterday that Ananias was living under a threat that no longer existed when he was asked to go to Saul. GOD had already entered in and changed the whole scenerio... the terrorist had changed without God letting Ananias know... and he was on STRAIGHT STREET! As I read the note about Daniel's vision changing the scripture rose inside: "if your eye be single it is full of light" and my heart soared! HE does make the crooked ways STRAIGHT and makes a way where there is no way! I am rejoicing with you and will continue to praise God for His wonderful work completed before we see it... calling those things not seen yet, but already opened-armed received... Love and blessings to your family today!

  6. Wow, Esther, Wow!So excited for the Plan. Love that God is moving in massive ways, and still manages to surprise us. Moved to tears again. So often I forget the enormity of this all for you, and the reality of it... but that you're living through it, grabbing hope and throwing that line to all around amazes me. I'm so excited to see what God does next, and am standing alongside you in prayer and declaring that this is the last of it... that there is restoration and healing, and this is only the beginning. What a beautiful picture of Daniel, eyes straight...
    Love you Esther, you and all your clan, so very much
    Beth xxx

  7. Faith, hope and love. The three greatest and most eternal of all things, you've had love and faith in huge dollops and inspired the pants off so many of us and it's so so so good to see you now have a lovely dollop of hope too. Standing with you every step of the way with our eyes fixed on our God who is watching and waiting around every blind corner,
    Huge huge love from the south,
    Rach V xxxx

  8. Yay!!!!!! I just want to run around, arms in the air and cheer!! :D xxxxxxxx

  9. We have been praying in our Bible Study Fellowship for the last few weeks.This is great to hear, we will keep praying for you all, we are so encouraged by your faith. Sarah x

  10. This is wonderfully amazing news and so, so deserved for you all. Tears flow yet again from reading your Blog but this time they are of joy. It is a privilege to pray with you for your little man and to pray for you and Richard to be sustained throughout this struggle. Love and Prayers Elaine x (you don't know me but I'm from Ribble Valley Church)

  11. Rejoycing much with you, in this new HOPE. Sending you our Love and continued prayers God is good. xx Jim and Joy xx

  12. Wonderful, wonderful news Esther. Our love and prayers are with you. Megan and Roy

  13. Hi, I wanted to introduce myself. My name is Kelly Marsh, and my daughter Hannah was diagnosed with HLH back in the summer of 2008. She was almost 6 years old at the time.

    I am happy to say that my daughter is a 3.5 year HLH/BMT survivor, and I just wanted to reach out and offer you support and to let you know there is hope, and there are survivors. I also urge you to connect with a group I created on Facebook called HLH Family (Survivors & Angels). You will find many families that can offer support and advice. There are also many DOCS that cover all things related to HLH, including medical notes taken from the latest HLH Conference, "What Every Family Needs to Know Heading Towards Transplant" and many others.

    I've also included the links to Hannah's You Tube video as well as her carepage.

    I realize this is a very direct approach from someone you've never met, but I've seen too many families go through this and not have the answers they are looking for. I was thrown into the HLH world over 3 years ago and I know how you all are feeling.

    You can call me anytime, day or night. The same goes for your family members. You do not have to go through this alone.

    Kelly Marsh
    Mom to and HLH/BMT Survivor
    My direct email is KelBelleFL@aol.com
    Hannah's website: www.carepages.com/carepages/hannahmarsh
    Hannah's Video: http://www.youtube.com/watch?v=A0I48X6YrDI

  14. Tears, smiles and continuing prayers. :D Deborah-Lynn xx

  15. Esther I'm so happy for you all that things are going according to plan. We're all thinking of you and hoping everything goes as smoothly as possible. As ever, if there's anything I can do... x

  16. My best friends 3year old brother has been suffering with hlh for the past two years. There have been a few poss transplants but for one reason or another they have fallen through. Its so refreshing to read your story and you are in my prayers. I will be directing said friend the way of your blog as at the moment its so hard to see the light at the end of the tonel x


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